Abstract
A 28-year-old. female developed high fever, anemia and splenomegaly. Abnormal laboratory findings including low hemoglobin concentration of 5.0 g/dl, reticulocytosis of 160‰, serum indirect bilirubin of 1.03 mg/dl, low haptoglobin of less than 5 mg/dl, low serum complement of 27 mg/dl C3 and 4 mg/dl C4, high cold agglutinin titers of ×2,048, positive direct Coombs test, the presence of anti I specificity on the red cells, and IgM (k) antibody identified in the extract of the washed red cells suggested the clinical diagnosis of cold agglutinin syndrome. Because of the lack of underlying diseases, the cold agglutinin syndrome in this patient was considered to be primary and of chronic type. Cyclophosphamide in combination with prednisolone brought some improvements of clinical manifestations.
